ewings sarcoma - dr. vandana
DESCRIPTION
Identified in 1921 by James Ewing2nd most common bone tumor in childrenEwing’s Sarcoma Family of tumors:Ewing’s sarcoma (Bone –87%) Extraosseous Ewing’s sarcoma (8%)Peripheral PNET(5%) Askin’s tumorTRANSCRIPT
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Ewing’s Sarcoma
Dr. VandanaDept of Radiotherapy,CSMMU, Lucknow
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Identified in 1921 by James Ewing 2nd most common bone tumor in children Ewing’s Sarcoma Family of tumors:
◦ Ewing’s sarcoma (Bone –87%) ◦ Extraosseous Ewing’s sarcoma (8%)◦ Peripheral PNET(5%) ◦ Askin’s tumor
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Introduction
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2% of cancer childhood malignancy Occurs most commonly in 2nd decade
◦ 80% occur between ages 5 and 25 M:F 1.3:1 < 10 yrs
1.6:1 > 10 yrs Rare in African-Americans and Asians
Epidemiology
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Pathology
One of many ‘small round blue cell’ tumors seen in pediatrics
Poorly differentiated tumor
Unknown origin, Thought to be of neural crest progenitor cells origin
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Consistent cytogenetic abnormality, t(11;22)(q24;q12) present in 90-95%◦ resultant fusion gene is EWS/FLI-1
Also seen:◦ t(21;22)(q22;q12) 5-10%
EWS/ERG◦ t(7;22) and t(17;22) the remainder
EWS/ETV1 and EWS/E1AF respectively◦ t(1;16)(q21;q13)
present along with t(11;22) The c-myc protooncogene is frequently expressed in Ewing’s. CD 99 ( MIC2) PAS +ve
Cytogenetics
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1
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Pain & swelling of affected area
May also have systemic symptoms:◦ Fever◦ Anemia◦ Weight loss ◦ Elevated WBC & ESR,LDH
Longest lag time in diagnosis for any pediatric solid tumor (mean of 146 days)
Pathological fracture
Clinical Presentation
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Location more common in diaphysis or
metadiaphysis
central axis (47%): ◦ pelvis, chest wall, spine, head &
neck
extremities (53%)
Scapula (3.8%)
Skull(3.8%)
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direct extension into adjacent bone or soft tissue. Metastases generally spread through bloodstream 25% present with metastatic disease
◦ Lungs (38%)◦ Bone (31%)◦ Bone Marrow (11%)
Nearly all pts. have micromets at diagnosis, so all Need chemo.
Routes of spread
No mets75%
Lu+Bone/BM 4 %
Lung 13%
Bone/BM 7 %
Other 1 %
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No uniform staging system.
The AJCC staging systems for bone or soft-tissue sarcomas may be used.
Staging
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Primary tumor (T)
TX Primary tumor cannot be assessed
T0 No evidence of primary tumor
T1 Tumor 8 cm or less in greatest dimension
T2 Tumor more than 8 cm in greatest dimension
T3 Discontinuous tumors in the primary bone site
Regional lymph nodes (N)
NX Regional lymph nodes cannot be assessed
N0 No regional lymph node metastasis
N1 Regional lymph node metastasis
Note: Because of the rarity of lymph node involvement in bone sarcomas, the designation NX may not be appropriate and cases should be considered N0 unless clinical node involvement is clearly evident.
Distant metastasis (M)
M0 No distant metastasis
M1 Distant metastasis
M1a Lung
M1b Other distant sites
AJCC Staging (7th Ed. 2010) Bone Tumor
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IA T1 N0 M0 G1,2 low grade, GX
IB T2 N0 M0 G1,2 low grade, GX
T3N0 M0 G1,2 low grade, GX
IIA T1 N0 M0 G3, 4 high grade
IIB T2 N0 M0 G3, 4 high grade
III T3 N0 M0 G3, 4IVA Any T N0 M1a any GIVB Any T N1 any M any G
Any T any N M1b any G
Stage
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Disease factors
Favorable prognosis
Unfavorable prognosis
Site Distal extremity (tibia, fibula, radius, ulna, hands, feet)
Central lesions (especially pelvic bones) less favorable: proximal extremity (humerus, femur), ribs
Size <8 cm in greatest diameter or <200 mL estimated volume
Larger tumors
Soft tissue extension
Absence of radiographically identifiable soft tissue extension
Presence of soft tissue extension by radiograph or significant extension by computed tomography
Extent of disease
Localized Metastatic
Site of Metastasis
Lung Bone / bone marrowBoth Lung and Bone
Response to CT
Responsive Unresponsive
Prognostic Factors
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Survival
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Primary Staging
History & Physical Examination
Histo-pathology -Biopsy-Genetics-IHC
-Bone Marrow
Imaging -X-ray-CT scan-MRI
-CT Thorax-Bone scan-PET scan
Lab Test - Renal – RFT - Cardiac – 2D-ECHO
Diagnostic Work-Up
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Confirmation of diagnosis:◦ biopsy and histopathologic examination
core needle / open Inx biopsy
◦ Cytogenetics and IHC
Biopsy
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Imaging
X-RAY◦ Moth eaten lesion◦ Lytic or mixed lytic-sclerotic
areas present◦ Multi-Layered subperiosteal
reaction (onion skinning)◦ Lifting of perioteum (codman’s
triangle)
CT SCAN: bone destruction best seen
Intramedullary space extraosseous involvement
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Involvement detected by MRI extends beyond the anticipated area seen on plain X-ray
Intra-medullary extent Soft tissue extension Skip lesions Relation Adjacent structures, vessels ,
nerves Multi-planar
MRI
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Bone scan: ◦ To detect polyostotic involvement◦ to detect bone metastasis
Bone marrow biopsy
CXR/CT of chest: lung mets
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Fig: bone scan shows increased activity in the distal femur.
Bone Scan: Ewing Sarcoma of Left Humerus demonstrates Intense Uptake
Gross Pathology: Ewing Sarcoma of Metadiaphysis of Proximal Humerus. (Top arrow) Permeative Marrow Lesion.(Bottom arrow) Surrounding Soft Tissue Mass
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newer technique Under evaluation to detect
◦ local and distal extent, ◦ Predictor of outcome and recurrence
PET/PET- CT Scan
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Laboratory tests: ◦ CBC, Alkaline phosphatase, liver/kidney function tests,◦ LDH:
useful as gauge of tumor burden Falls with effective therapy and rises with disease
recurrence
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Multidisciplinary approach
◦ Chemotherapy: control of micrometasis
◦ Surgery: local control where possible
◦ Radiotherapy: local control where surgery not possible or . incomplete
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Treatment
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Effective local and systemic chemotherapy necessary for cure.
Induction chemotherapy preferred over starting the systemic and local therapy
Advantage of this approach:◦ Evaluation of effectiveness of the regimen◦ Decreases the vol. of local therapy for surgery or
RT◦ Some bone healing occurs during CT, diminish the
risk of pathological fracture
General Management
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Induction Chemotherap
y
Local Control• Surgery• Radiothera
py
Maintenance• Chemother
apy
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Chemotherapy
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All patients require chemotherapy◦ Induction chemotherapy◦ Maintenance chemotherapy
Effective chemotherapy has improved local control rates achieved with radiation to 85-90%
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Chemotherapy
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First Line therapy:◦ VAC/IE
Vincristine 2.0 mg/m2 on D1 Adriamycin 75 mg/m2 on D1 Cyclophosphamide 1.2 gm/m2 on D1 Ifosphamide 1.8 gm/m2 on D1-5 Etoposide 100 mg/m2 on D1-5
◦ **Substitute adriamycin with dactinomycin (1.2 mg/m2 on D1) after 375 mg/m2
◦ VAI (Vincristine, Adriamycin, Ifosphamide)◦ VIDE ( Vincristine, ifosphamide, Doxorubicin, Etoposide)
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Chemotherapy
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Cyclophosphamide (250 mg/m2)and topotecan(0.75 mg/m2) D1-D5
Temozolomide and irinotecan Ifosfamide and etoposide Ifosfamide ,etoposide and carboplatin Docetaxel and gemcitabine
Second line therapy (relapse and refractory disease)
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IESS-1and IESS-2 showed 4 drug regimen VACD is superior to 3 drug VAC in terms of RFS and OS.
INT-OO91:Adding IE improved 5-year OS (61→72%) for localized disease, but not for metastatic disease (25%).
Clinical Studies
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Induction Multiagent chemotherapy for at least 12-24 weeks prior to local therapy.
Maintenance (adjuvant chemotherapy) with or without Radiotherapy is recommended following local control treatment and the duration of chemotherapy should be between 28-49 weeks.
**NCCN guidelines version 2.2012
DURATION
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Surgery
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Development of Innovative Surgical Techniques: Limb preservation & Structural bone function preservation
Chemo - cytoreduction makes resection possible
Local failure rates with RT in historical series : 9 - 25% * Concern over second malignancies
* Horonitz et al, Pediatr Clin Nor Am, 1991
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Role of Surgery
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Surgical Indications◦ Expendable bone (fibula, rib, clavicle)◦ Bone defect able to be reconstructed with modest loss of
function◦ May consider amputation if considerable growth remaining◦ After pre-op RT
Limb-salvage surgery is preffered. Curative surgery requires wide local excision and
negative margin◦ Bony margins of at least 1 cm, with a 2 to 5 cm margin
recommend.◦ Soft tissue at least 5mm in fat or muscle , with 2mm through
fascial planes.
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Radiotherapy
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radiation responsive tumor.
There are no randomized trials that have directely compared Radiotherapy to surgery for local control of Ewing’s sarcoma.
Radiotherapy can, in combination with chemotherapy, achieve local control, but complete surgery when feasible has to be regarded as the first choice of local therapy.**
**ESMO clinical practice Guidelines for diagnosis, treatment and follow-up for Bone sarcomas. Ref. Annals of Oncology 21 (Supplement 5) 13,2010
Radiotherapy
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Radiotherapy detail Patient may be treated in supine ,prone, or
lateral position site dependent. 6MV of energy used For limb, opposing fields normally used. Tailored portals for every patient. Field should not cross joints unless essential. Entire Medullary cavity need not be included
in the RT portal. Try and spare a strip(1-2cm) of normal tissue
for lymph drainage.
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FIG. Changes in treatment volume. (A) Field encompassing the entire length of the medullary cavity for a tumor involving the proximal left humerus. (B) Tailored field encompassing only the proximal aspect of the leg for a limited tumor of the left tibia.
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Definitive Radiation Therapy:
◦ Tumors where Resection is Impossible ◦ For skull, face, vertebra, or pelvic primary◦ where only an intra-lesional resection is achievable◦ Patient with poor Surgical risk◦ Patient refusing surgery
Note: Surgery is the preferred arm where wide or marginal resection is possible
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Indications for RT: After induction chemotherapy
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Pre-operative Radiation Therapy
◦ Indicated when narrow resection margins are expected
◦ Principle :
To sterilize the tumor compartment before surgery & to
potentially reduce the risk of dissemination during
surgery
◦ Local recurrence with pre-op RT
<5% EI-CESS-92 : Schuck et al – IJROBP-1998 & 2003
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Cont…
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Post-operative Radiation Therapy
◦ For gross or microscopic positive margin◦ For marginal Resection◦ For wide-resection with Poor Histological response to
Neo-adjuvant Chemotherapy (>10% viable tumor cells in the specimen)
Based on CESS-81, CESS-86, EICESS-92 Studies : Schuck et al,IJROBP-1998 & 2003
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Cont…
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Definitive RT◦ Phase 1:
Gross tumor in bone and soft tissue (pre chemo ) + 2-4 cm longitudinal margins + 2 cm lateral margins.
Dose:45 Gy/180cGy/#
◦ Boost phase : Reduced 1-2 cm margins(bone and residual tissue) Up to total dose of 55.8Gy.
Note: - In case of no soft tissue involvement, the proximal and distal margins in bone are not changed.
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Planning
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Figure: Schematic depiction of GTV1 (pre-induction bone and pre-induction soft tissue extent) and GTV2 (pre-induction and post-induction soft tissue extent)
Pre-chemotherapy tumor Post-chemotherapy tumor
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Pretreatment gross tumor volume +surgical scar+2cm margin(45 Gy) boost to post op residual +2cm margin.
Dose: ◦ MICROSCOPIC DISEASE- 45 Gy◦ MACROSCOPIC RESIDUAL – 55.8Gy
Pre op RT 45 Gy to original bone and soft tissue
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Post-op RT Planning
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For rib primary ,with pleural effusion, RT to hemithorax
For lung mets ,whole lung RT(15-18 Gy) or consider resection if< 4 mets.
Pain palliation– advanced disease.
Isolated bone secondaries.
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Palliative RT
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Clinical Situation Total Dose (%)
Dose per Fraction (%)
Gross disease (after biopsy only or intralesional resection)
1. Treatment once a day
Initial Field
45 1.8
Boost field 10.8 1.8
After marginal resection or poor histologic response at surgery
41.4 – 45 1.8
Preoperative radiotherapy:
45 1.8
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Recommendations for RT Fields & doses
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If disease extension into pre-formed body cavities e.g. lung & pelvis, radiotherapy volume includes post induction volume with 2cm margin in order to reduce treatment related toxicity.
Lesion of vertebral body treated with 45Gy to 50.4Gy
More than 20 Gy can prematurely close epiphysis.
20–30 Gy usually can be given to entire circumference of an extremity, doesn’t cause lymphedema.
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Physical Exam, Local and Chest Imaging:
• Every 2- 3 months• Increase interval after 24 months• Annually after 5 years indefinitely
CBC and other lab studies as indicated
Consider Bone Scan or Pet scan
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Surveillance
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30-40% of patients develop relapse with <20% survival
Early relapse – less than 2 years: Consider Changing Chemotherapy
Late relapse – more than 2 years: Continue the previously used chemotherapy
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Relapse
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Functional results : Of all the patient’s treated with RT ◦ 60 % have good functional activity◦ 20 % have mild morbidities◦ 20 % have significant morbidities
Risk for Post treatment Fractures Lymphedema Dermatitis; recall reaction may occur with
doxo, dactinomycin. Adriamycin cardiomyopathy. Ifosphamide renal toxicity.
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Side Effects
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Second malignancy after RT◦ Cumulative risk at 15yrs = 6 – 6.7%
( CESS-81 & CESS-86; IJROBP:1997; 39) ◦ No secondary sarcomas seen at doses <48 Gy
( Kutterch et al; JCO:1996, 14 ) ◦ Risk increased by anthracycline and alkylating
agent chemotherapy ◦ Osteosarcoma most common.◦ Leukemia can also occur.
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Side Effects
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Use of 3D-CRT / IMRT as a standard protocol
Incorporation of functional imaging modalities e.g. PET-CT / PET-MRI for Target Volume delineation, Boost treatment and IMRT
TARGATED therapy :Molecular agents like Apoptosis directed targeted therapies e.g. TRAIL therapy (TNF Related Apoptosis Inducing Ligand),anti IGF-1R antibodies…etc
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In the near Future
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Overview of Ewing’s tumor treatment
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Second most common childhood bone tumor. Small round cell tumor with CD99 (MIC2), PAS positive Lytic lesion with onion peel appearance on X-Ray Overall survival with localized disease (55%) and
metastatic disease 22% Multimodal treatment approach Induction Chemotherapy for 3-6 cycles and another 6-10
cycles for maintenance. Surgery when feasible first choice of local therapy Radiation responsive tumor There are no randomized trials that have directely
compared Radiotherapy to surgery for local control of Ewing’s sarcoma.
Conclusion
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Thank You